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https://www.readbyqxmd.com/read/29326878/ten-year-long-enzyme-replacement-therapy-shows-a-poor-effect-in-alleviating-giant-leg-ulcers-in-a-male-with-fabry-disease
#1
Jun Okada, Mohammad Arif Hossain, Chen Wu, Takashi Miyajima, Hiroko Yanagisawa, Keiko Akiyama, Yoshikatsu Eto
Fabry disease is an X-linked lysosomal storage disorder caused by a deficiency of α-galactosidase A (α-gal A), leading to the progressive accumulation of glycosphingolipids. Classical hemizygous males usually present symptoms, including pain and paresthesia in the extremities, angiokeratoma, hypo- or anhidrosis, abdominal pain, cornea verticillata, early stroke, tinnitus, and/or hearing loss, during early childhood or adolescence. Moreover, proteinuria, renal impairment, and cardiac hypertrophy can appear with age...
March 2018: Molecular Genetics and Metabolism Reports
https://www.readbyqxmd.com/read/29321204/larval-infestation-of-chronic-ischaemic-leg-ulcer
#2
Walid Ibrahim, Lubna Osman, Ahmed Subahi
No abstract text is available yet for this article.
January 9, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29320975/systemic-lupus-erythematosus-with-inflammatory-bowel-disease-ulcerative-colitis-case-report
#3
H Elsayed Mansour, S G Arafa, W Abdelfatah Shehata
A 30-year-old female presented to the rheumatology outpatient clinic of the Internal Medicine Department, Ain Shams University Hospital, Cairo, Egypt, complaining of a large right leg ulcer consistent with pyoderma gangrenosum. There was history of recurrent attacks of bleeding per rectum of one-year duration. During hospitalization she noticed blurring of vision in the left eye with diffuse blackish discoloration of the feet and toes, consistent with small-vessel vasculitis. Colonoscopy with biopsy and histopathology confirmed the diagnosis of inflammatory bowel disease-ulcerative colitis (IBD-UC)...
January 1, 2018: Lupus
https://www.readbyqxmd.com/read/29320522/do-systematic-reviews-address-community-healthcare-professionals-wound-care-uncertainties-results-from-evidence-mapping-in-wound-care
#4
Janice Christie, Trish A Gray, Jo C Dumville, Nicky A Cullum
BACKGROUND: Complex wounds such as leg and foot ulcers are common, resource intensive and have negative impacts on patients' wellbeing. Evidence-based decision-making, substantiated by high quality evidence such as from systematic reviews, is widely advocated for improving patient care and healthcare efficiency. Consequently, we set out to classify and map the extent to which up-to-date systematic reviews containing robust evidence exist for wound care uncertainties prioritised by community-based healthcare professionals...
2018: PloS One
https://www.readbyqxmd.com/read/29319041/successful-pulmonary-thromboendarterectomy-in-a-patient-with-sickle-cell-disease-and-associated-resolution-of-a-leg-ulcer
#5
Abhinav Agrawal, Rakesh Shah, Matthew D Bacchetta, Arunabh Talwar
Pulmonary hypertension (PH) is a relatively frequent and severe complication of sickle cell disease (SCD). PH associated with SCD is classified as Group 5 PH. The exact pathogenesis of PH in SCD in not known. There are also very limited treatment options available at this time for such patients with Group 5 PH. Patients with SCD are predisposed to a hypercoagulable state and thus can also suffer from chronic thromboembolism. These patients can have associated chronic thromboembolic pulmonary hypertension (CTEPH), thus being classified as Group 4 PH...
January 2018: Lung India: Official Organ of Indian Chest Society
https://www.readbyqxmd.com/read/29317879/clinical-features-of-anthroponotic-cutaneous-leishmaniasis-in-a-major-focus-southeastern-iran-1994-2014
#6
Ahmad Khosravi, Iraj Sharifi, Alireza Fekri, Alireza Kermanizadeh, Mehdi Bamorovat, Mahshid Mostafavi, Mohammad Reza Aflatoonian, Alireza Keyhani
Background: Cutaneous leishmaniasis (CL) is associated with a broad and complex clinical spectrum of diseases. The objectives of this study were to assess the clinical features and identification of the causative agents of CL in a well-known focus of anthroponotic CL (ACL) caused by Leishmania tropica, southeast Iran. Methods: This study was performed randomly as a descriptive cross-sectional survey to evaluate 2000 CL patients by active and passive case-detection approaches in Kerman Province from 1994 to 2014...
October 2017: Iranian Journal of Parasitology
https://www.readbyqxmd.com/read/29317214/case-series-use-of-glyceryl-trinitrate-patches-to-improve-peripheral-circulation-in-children-with-severe-neurodisability
#7
Frances Morrison, Jack Fairhurst, Charlie Fairhurst
AIM: Evaluation of topical Glyceryl trinitrate as a therapeutic option to improve peripheral circulation in 16 children with severe complex neurodisability, poor capillary return and signs of vascular insufficiency, including discomfort. METHODS: If insufficient improvement in capillary return was obtained using conservative measures, a 5 mg GTN patch was placed each day on the front of the tibia of each leg. Children were reviewed clinically for up to 12 months of treatment...
December 31, 2017: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/29316681/non-transfusion-dependent-thalassemia-an-update-on-complications-and-management
#8
REVIEW
Joseph Sleiman, Ali Tarhini, Rayan Bou-Fakhredin, Antoine N Saliba, Maria Domenica Cappellini, Ali T Taher
Patients with non-transfusion-dependent thalassemia (NTDT) experience many clinical complications despite their independence from frequent transfusions. Morbidities in NTDT stem from the interaction of multiple pathophysiological factors: ineffective erythropoiesis, iron overload (IOL), and hypercoagulability. Ineffective erythropoiesis and hemolysis are associated with chronic hypoxia and a hypercoagulable state. The latter are linked to a high prevalence of thromboembolic and cerebrovascular events, as well as leg ulcers and pulmonary hypertension...
January 8, 2018: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/29301794/surgical-trauma-induces-overgrowth-in-lower-limb-gigantism-regulation-with-use-of-rapamycin-is-promising
#9
Rohan Sebastian Pinto, William David Harrison, Kenneth Graham, Durai Nayagam
We describe an unclassified overgrowth syndrome characterised by unregulated growth of dermal fibroblasts in the lower limbs of a 35-year-old woman. A PIK3CA gene mutation resulted in lower limb gigantism. Below the waist, she weighed 117 kg with each leg measuring over 100 cm in circumference. Her total adiposity was 50% accounted for by her legs mainly. Liposuction and surgical debulking were performed to reduce the size of the limbs but had exacerbated the overgrowth in her lower limbs. Systemic sepsis from an infected foot ulcer necessitated treatment by an above-knee amputation...
January 4, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29301491/pancreatic-panniculitis-the-bright-side-of-the-moon-in-solid-cancer-patients
#10
Elena Guanziroli, Antonella Colombo, Antonella Coggi, Raffaele Gianotti, Angelo Valerio Marzano
BACKGROUND: Pancreatic panniculitis is a rare complication of pancreas disorders occurring in 0.3-3% of patients, most often accompanied by the pancreatic acinar carcinoma. It presents multiple, painful, deep, ill-defined, red-brown, migratory nodules and plaques of hard elastic consistency; often ulcerated and typically located on the lower proximal and distal extremities. The pathogenesis is not fully understood, but it is thought to result from lipolysis and fat necrosis with secondary tissue inflammation induced by pancreatic enzymes...
January 4, 2018: BMC Gastroenterology
https://www.readbyqxmd.com/read/29295792/sickle-cell-retinopathy-and-other-chronic-complications-of-sickle-cell-anemia-a-clinical-study-of-84-sub-saharan-african-cases-cameroon
#11
Y Bilong, M Dubert, G Koki, J J Noubiap, H N Pangetna, A Menet, D Chelo, L Offredo, S Jacob, S Belinga, A N A Yanda, S Kingue, X Jouven, B Ranque, L A Bella
OBJECTIVE: Sickle retinopathy is a severe complication of sickle cell disease than can lead to blindness. We aim to describe the epidemiology of sickle retinopathy in homozygous sickle cell (SS) African patients and to analyze its association with non-ophthalmologic disease complications of sickle cell anemia. METHODS: We conducted a nested study within the CADRE cohort in Cameroon. Eighty-four consecutive SS outpatients, aged 10 years and older, with no visual symptoms, underwent an ophthalmologic examination...
December 30, 2017: Journal Français D'ophtalmologie
https://www.readbyqxmd.com/read/29285959/kyrle-s-disease-effectively-treated-with-oral-isotretinoin
#12
Martina Maurelli, Paolo Gisondi, Giampiero Girolomoni
BACKGROUND: Kyrle's disease is a primary perforating dermatosis that affects more commonly 30-50 year old females, and clinically characterized by pruritic hyperkeratotic and ulcerated nodules, papules and plaques, localized on extensor surface of upper and lower limbs, and on the trunk. OBJECTIVE: To determine the effectiveness of the treatment with oral isotretinoin in Kyrle's disease. METHODS: We present the case of a 63-year-old woman with hyperkeratotic, erythematous-brown nodules and plaques localized on arms and legs, intensely itching; some lesions were ulcerated and discharging...
December 29, 2017: Journal of Dermatological Treatment
https://www.readbyqxmd.com/read/29277969/predicting-delayed-healing-the-diagnostic-accuracy-of-a-venous-leg-ulcer-risk-assessment-tool
#13
Helen E Edwards, Christina N Parker, Charne Miller, Michelle Gibb, Suzanne Kapp, Rajna Ogrin, Jacinta Anderson, Kerrie Coleman, Dianne Smith, Kathleen J Finlayson
The aim of this study was to validate a newly developed tool that can predict the risk of failure to heal of a venous leg ulcer in 24 weeks. The risk assessment tool was validated, and performance of the tool was assessed using Area Under the Receiver Operating Characteristic Curve (AUC) analysis. Retrospective and prospective validation was conducted through multi-site, longitudinal studies. In the retrospective study (n = 318), 30% of ulcers did not heal within 24 weeks, with the tool demonstrating an AUC of 0...
December 26, 2017: International Wound Journal
https://www.readbyqxmd.com/read/29277134/customizing-elastic-pressure-bandages-for-reuse-to-a-predetermined-sub-bandage-pressure-a-randomized-controlled-trial
#14
Nuttawut Sermsathanasawadi, Tanakorn Tarapongpun, Rattana Pianchareonsin, Nattawut Puangpunngam, Chumpol Wongwanit, Khamin Chinsakchai, Pramook Mutirangura, Chanean Ruangsetakit
Objective A randomized clinical trial was performed to compare the effectiveness of unmarked bandages and customized bandages with visual markers in reproducing the desired sub-bandage pressure during self-bandaging by patients. Method Ninety patients were randomly allocated to two groups ("customized bandages" and "unmarked bandages") and asked to perform self-bandaging three times. The achievement of a pressure between 35 and 45 mmHg in at least two of the three attempts was defined as adequate quality...
January 1, 2017: Phlebology
https://www.readbyqxmd.com/read/29276458/association-of-venous-leg-ulcers-with-ankle-range-of-motion-in-people-attending-chiropractic-mobile-clinics-in-the-dominican-republic
#15
Patricia A Tavares, Victoria Landsman, Nathalie Gomez, Ariel Ferreiras, Ramon A Lopez
Objective: The goal of the study was to determine if there was an association between chronic venous disorders (CVDs), particularly venous leg ulcers, and ankle range of motion (ROM) in the Dominican Republic. Methods: Chronic venous disorders were classified using the clinical manifestations portion (C) of the CEAP (clinical manifestations, etiology, anatomy, pathophysiology) method. The legs of participants attending mobile chiropractic clinics in rural, low-income areas in the Dominican Republic were assessed for clinical signs of CVD and venous ulcers...
December 2017: Journal of Chiropractic Medicine
https://www.readbyqxmd.com/read/29260248/-chronic-refractory-ulcer-on-the-ring-finger-manifestation-of-distal-epithelioid-sarcoma
#16
N S Häring, R Strohal, S Dertinger, F Offner
The epithelioid sarcoma classic, "distal" type was first published in 1970. It is a very rare, malignant, aggressive subcutaneous soft tissue sarcoma that shows characteristic positivity for both epithelial and mesenchymal immunohistochemical markers. It grows very slowly and mostly presents in young men. Clinically the tumor is characterized as a coarse cutaneous or subcutaneous nodular induration that often ulcerates in the course of the disease. An association with trauma is often described and can lengthen time to diagnosis...
December 19, 2017: Der Hautarzt; Zeitschrift Für Dermatologie, Venerologie, und Verwandte Gebiete
https://www.readbyqxmd.com/read/29251541/imagej-a-free-easy-and-reliable-method-to-measure-leg-ulcers-using-digital-pictures
#17
Javier Aragón-Sánchez, Yurena Quintana-Marrero, Cristina Aragón-Hernández, María José Hernández-Herero
Wound measurement to document the healing course of chronic leg ulcers has an important role in the management of these patients. Digital cameras in smartphones are readily available and easy to use, and taking pictures of wounds is becoming a routine in specialized departments. Analyzing digital pictures with appropriate software provides clinicians a quick, clean, and easy-to-use tool for measuring wound area. A set of 25 digital pictures of plain foot and leg ulcers was the basis of this study. Photographs were taken placing a ruler next to the wound in parallel with the healthy skin with the iPhone 6S (Apple Inc, Cupertino, CA), which has a camera of 12 megapixels using the flash...
December 2017: International Journal of Lower Extremity Wounds
https://www.readbyqxmd.com/read/29250935/identifying-risk-factors-associated-with-infection-in-patients-with-chronic-leg-ulcers
#18
Ut T Bui, Helen Edwards, Kathleen Finlayson
Leg ulcers are hard to heal. Infection causes delayed healing, negatively impacting patients' quality of life, the healthcare system, and society. Early recognition of patients at high risk of infection is essential to prevent complications and reduce negative impacts. However, at present, factors associated with infection in this population are not yet clearly understood. The study aimed to identify factors that were significantly associated with infection in chronic leg ulcers. A sample of 561 patients with chronic leg ulcers, who were previously recruited at outpatient clinics and community settings within Australia between 2008 and 2015, were selected for the current analysis...
December 17, 2017: International Wound Journal
https://www.readbyqxmd.com/read/29248102/the-center-for-vein-restoration-study-on-presenting-symptoms-treatment-modalities-and-outcomes-in-medicare-eligible-patients-with-chronic-venous-disorders
#19
Peter J Pappas, Sanjiv Lakhanpal, Khanh Q Nguyen, Rohan Vanjara
BACKGROUND: Chronic venous disorders (CVDs) have been estimated to affect up to 20 million Americans. Despite this huge prevalence, the signs, symptoms, and treatment outcomes in patients 65 years of age and older are not well defined. Our goal was to determine the presentation and treatment outcomes in elderly patients compared with a cohort of patients younger than 65 years. METHODS: From January 2015 to December 2016, we retrospectively reviewed prospectively collected data from 38,750 patients with CVD from the Center for Vein Restoration's electronic medical record (NextGen Healthcare Information Systems, Irvine, Calif)...
January 2018: Journal of Vascular Surgery. Venous and Lymphatic Disorders
https://www.readbyqxmd.com/read/29245283/successful-treatment-of-erythema-induratum-with-topical-application-of-antituberculous-drugs-a-case-report
#20
Xueling Mei, Junying Zhao
RATIONALE: Erythema induratum, a chronic recurrent lobular panniculitis with vasculitis, is strongly associated with Mycobacterium tuberculosis infection. The recommended drugs include isoniazid, rifampicin, and pyrazinamide, which are typically administered in combination (orally or intravenously). Till date, there are no reports about topical application of anti-tuberculous (anti-TB) drugs for treatment of erythema induratum. PATIENT CONCERNS: Herein, we present the case of a 73-year-old woman with recurrent ulceration, scarring and obvious pain in her lower legs...
December 2017: Medicine (Baltimore)
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